Pranati Mohanty1, Rabindra Kumar Jena2, Sudha Sethy3. 1. Assistant Professor, Department of Pathology, S.C.B. Medical College and Hospital, Cuttack, Odisha, India. 2. Professor and Head, Department of Clinical Haematology, S.C.B. Medical College and Hospital, Cuttack, Odisha, India. 3. Assistant Professor, Department of Clinical Haematology, S.C.B. Medical College and Hospital, Cuttack, Odisha, India.
Abstract
INTRODUCTION: Sickle Cell Anaemia (SCA) is one of the commonest haemoglobinopathies due to a point mutation (A→T) of the β-globin gene. Out of five haplotypes, the Arab-Indian haplotype present in India is one of the least severe phenotype and least studied also. It is characterized by lifelong haemolytic anaemia requiring red cell transfusion leading to iron overload. In contrast, there is very high incidence of deficiency of iron, folic acid and vitamin B12. AIM: Our objective was to access the Iron status of SCA patients and to find its correlation with various parameters like red cell transfusion, haemolysis and serum hepcidin. MATERIALS AND METHODS: This was a cross-sectional study conducted on 208 patients for a period of five years. Complete Blood Count (CBC), iron profile, haemolytic parameters and transfusion requirement were studied and data compared with 52 healthy controls. RESULTS: Few patients (9.6%) revealed significant iron overload (Serum ferritin > 1000 ng/ml). In majority (80.8%) it was either normal or border line raised (300 to 1000 ng/ml) or iron deficiency was noted in a small fraction (9.6%). Frequency of transfusion is the principal factor which positively correlated with level of iron load (p<0.001) while parameters of haemolysis and serum hepcidin level play an insignificant role in this context (p= 0.0634). CONCLUSION: This study supports the notion that the presentation of SCA patients in India is of "Viscosity - Vaso-Occlusive Crisis (VOC) phenotype" with high incidence of VOC, low haemolytic rate and transfusion requirement. Iron deficiency may be present in SCA patients requiring Iron supplementation. We suggest further studies to establish the role of hepcidin, ferroportin and other factors that control iron absorption in these patients.
INTRODUCTION:Sickle Cell Anaemia (SCA) is one of the commonest haemoglobinopathies due to a point mutation (A→T) of the β-globin gene. Out of five haplotypes, the Arab-Indian haplotype present in India is one of the least severe phenotype and least studied also. It is characterized by lifelong haemolytic anaemia requiring red cell transfusion leading to iron overload. In contrast, there is very high incidence of deficiency of iron, folic acid and vitamin B12. AIM: Our objective was to access the Iron status of SCApatients and to find its correlation with various parameters like red cell transfusion, haemolysis and serum hepcidin. MATERIALS AND METHODS: This was a cross-sectional study conducted on 208 patients for a period of five years. Complete Blood Count (CBC), iron profile, haemolytic parameters and transfusion requirement were studied and data compared with 52 healthy controls. RESULTS: Few patients (9.6%) revealed significant iron overload (Serum ferritin > 1000 ng/ml). In majority (80.8%) it was either normal or border line raised (300 to 1000 ng/ml) or iron deficiency was noted in a small fraction (9.6%). Frequency of transfusion is the principal factor which positively correlated with level of iron load (p<0.001) while parameters of haemolysis and serum hepcidin level play an insignificant role in this context (p= 0.0634). CONCLUSION: This study supports the notion that the presentation of SCApatients in India is of "Viscosity - Vaso-Occlusive Crisis (VOC) phenotype" with high incidence of VOC, low haemolytic rate and transfusion requirement. Iron deficiency may be present in SCApatients requiring Iron supplementation. We suggest further studies to establish the role of hepcidin, ferroportin and other factors that control iron absorption in these patients.
Entities:
Keywords:
Arab- Indian haplotype; Hepcidin; Point mutation; Red cell transfusion; Serum ferritin
Authors: Sara Gardenghi; Maria F Marongiu; Pedro Ramos; Ella Guy; Laura Breda; Amy Chadburn; YiFang Liu; Ninette Amariglio; Gideon Rechavi; Eliezer A Rachmilewitz; William Breuer; Z Ioav Cabantchik; Diedra M Wrighting; Nancy C Andrews; Maria de Sousa; Patricia J Giardina; Robert W Grady; Stefano Rivella Journal: Blood Date: 2007-02-13 Impact factor: 22.113
Authors: D Mohanty; M B Mukherjee; R B Colah; M Wadia; K Ghosh; G P Chottray; D Jain; Y Italia; K Ashokan; R Kaul; D K Shukla; V Muthuswamy Journal: Indian J Med Res Date: 2008-04 Impact factor: 2.375
Authors: Akinsegun A Akinbami; Adedoyin O Dosunmu; Adewumi A Adediran; Olajumoke O Oshinaike; Vincent O Osunkalu; Sarah O Ajibola; Olanrewaju M Arogundade Journal: J Blood Med Date: 2013-05-22
Authors: Osheiza Abdulmalik; Noureldien H E Darwish; Vandhana Muralidharan-Chari; Maii Abu Taleb; Shaker A Mousa Journal: Haematologica Date: 2022-02-01 Impact factor: 9.941